This document provides an overview of common pediatric solid tumors, including definitions, epidemiology, etiology, types, symptoms and signs, staging, diagnosis, treatment, and prognosis for each tumor type. The tumors discussed include lymphomas, nephroblastoma (Wilms tumor), neuroblastoma, hepatoblastoma, and hemangioma. For each tumor type, the summary defines the tumor, discusses prevalence and risk factors, describes common subtypes, and outlines the standard diagnostic and treatment approaches.
5. Definition
• Neoplasia of the lymphatic system and its precursor cells
with genetically distrusted regulation of proliferation,
differentiation and apoptosis.
• Characterized by painless, progressive enlargement of
the lymph nodes with continues extension between
lymph node regions.
6. Epidemiology
• Lymphoma accounts 10% of all pediatric neoplasia.
Hodgkin's lymphoma Non-Hodgkin's lymphoma
7 in 1 million children < age
of 16 newly diagnosed with
HL annually
Ratio of boys to girls 2:1
Boys > Girls , but during
adolescence the incidence
is the same
Peak incidence between 5
to 15 years old
Peak incidence between 15
to 35 years old
7. Etiology
• Unknown etiology in humans.
• It has been found that there is
correlation with infection “e.g
Epstein-barr Virus”.
• Correlation with
Socioeconomic status: the
higher SES the more
frequently HL occur.
• Genetic causes.
• Unknown etiology in humans.
• Predisposing factors include:
– Autoimmune disorders.
– Infections ( HIV, EBV, HTLV-1 )
– Congenital B-Cell defect.
– Congenital T-Cell defect.
– Drug induced
(immunosuppressive treatment)
HL NHL
13. Treatment
• Treatment plan is assigned based on
the type and stage of lymphoma.
• Traditionally, management consists of
a combination of chemotherapy
drugs.
• Combination of radiotherapy and
chemotherapy can be used in
Hodgkin's lymphoma.
18. Etiology
• Wilms tumor has been associated with loss of function mutations of
a number of tumor suppressor and transcription genes.
• These include mutations of the WT1, p53, FWT1, and FWT2 genes,
and at the 11p15.5 locus.
• Associated with some congenital syndromes. These syndromes
include:
– WAGR syndrome.
– Denys-Drash syndrome.
– Beckwith-Wiedemann syndrome.
24. Treatment
• Treatment plan is assigned based on results of the initial
staging, histological, and molecular studies.
• In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy
27. Definition
• Malignant embyronal tumor of precursor cells of sympathetic
ganglia and adrenal medulla.
• Commonly used to refer to a spectrum of neuroblastic tumors
(including neuroblastomas, ganglioneuroblastomas, and
ganglioneuromas)
29. Etiology
• Etiology is unknown.
Risk factors:
• Maternal factors: These include the following:
– Opiate consumption
– Folate deficiency
– Toxic exposures
– Congenital abnormalities
– Gestational diabetes mellitus
• Genetic factors:
– A higher incidence of neuroblastoma has been suggested in girls with Turner
syndrome, Hirschsprung's disease, central hypoventilation, and neurofibromatosis type 1
(NF1)
30. Types
• Neuroblastomas can arise anywhere throughout the
sympathetic nervous system.
• The common primary sites:
Adrenal gland
(40%)
Abdominal
(25%)
Thoracic
(15%)
35. Diagnosis
• Diagnostic criteria — Minimum criteria for establishing a diagnosis
of neuroblastoma have been agreed upon by an international
consensus panel. A definitive diagnosis of neuroblastoma requires
one of the following:
– An unequivocal histologic diagnosis from tumor tissue by light microscopy, with
or without immunohistochemistry, electron microscopy, or increased urine (or
serum) catecholamines or their metabolites.
– Evidence of metastases to bone marrow on an aspirate or trephine
biopsy with concomitant elevation of urinary or serum catecholamines or their
metabolites.
36. Treatment
• Treatment plan is assigned based on:
– Stage of the disease.
– Patient age.
– Histologic appearance of the tumor.
– Presence or absence of amplification of the MYCN oncogene.
– Quantitative DNA content of the tumor (DNA index or ploidy).
40. Definition
• Hepatoblastoma is the most common primary
hepatic malignancy in early childhood.
• The majority of hepatoblastomas occur in the
first two years of life and rarely in children older
than five years.
41. Epidemiology
• One percent of all pediatric neoplasias
• The incidence of hepatoblastoma in boys is
twice that in girls.
42. Etiology
• Exact etiology is unknown.
• Syndromes with an increased incidence of
hepatoblastoma include:
– Beckwith Wiedmann syndrome.
– Trisomy 18 & 21.
– Acardia syndrome.
– Li-Fraumeni syndrome.
– Goldenhar syndrome (a type of craniofacial microsomia).
– Type 1a glycogen storage disease (von Gierke’s disease).
– Familial adenomatous polyposis (FAP).
45. Staging
Stage Characteristics
Stage I • The tumor is completely resectable via
wedge resection or lobectomy.
• The tumor has PFH results.
• The AFP level is within reference range
within 4 weeks of surgery.
Stage IIA • The tumor is completely resectable.
• The tumor has histologic results other than
PFH (UH).
Stage IIB • The tumor is completely resectable.
• AFP findings are negative at time of diagnosis
(ie, no marker to follow).
Stage III (any of the following) • The tumor is initially unresectable but is
confined to one lobe of liver.
• Gross residual disease is present after
surgery.
• Tumor ruptures or spills preoperatively or
intraoperatively.
• Regional lymph nodes are involved.
Stage IV Distant bone or lung metastasis is present.
47. Treatment
• Treatment plan is assigned based on results of the initial
staging, histological, and molecular studies.
• In General , lines of treatment include:
Surgery Chemotherapy Radiotherapy
50. Definition
• Infantile hemangiomas are benign vascular neoplasms
that have a characteristic clinical course marked by early
proliferation and followed by spontaneous involution.
• Hemangiomas are the most common tumors of infancy
and usually are medically insignificant.
51. Treatment
• The vast majority of infantile hemangiomas do not
require any medical or surgical intervention.
• Intervention may be required for lesions with potential to
interfere with a vital structure or function. These include,
but are not limited to:
– Lesions in the airway, liver, or gastrointestinal tract.
– Lesions in the periorbital region.
– Very large, rapidly growing cutaneous hemangiomas.
The adrenal gland is the most common primary site (40 percent), followed by abdominal (25 percent), thoracic (15 percent), cervical (5 percent), and pelvic sympathetic ganglia (5 percent)
stage I
The tumor is completely resectable via wedge resection or lobectomy.
The tumor has PFH results.
The AFP level is within reference range within 4 weeks of surgery.
Stage IIA
The tumor is completely resectable.
The tumor has histologic results other than PFH (UH).
Stage IIB
The tumor is completely resectable.
AFP findings are negative at time of diagnosis (ie, no marker to follow).
Stage IIC
The tumor is completely resected or rendered completely resectable by initial radiotherapy or chemotherapy or microscopic residual disease is present.
The AFP level is elevated 4 weeks after resection.
Stage III (any of the following)
The tumor is initially unresectable but is confined to one lobe of liver.
Gross residual disease is present after surgery.
Tumor ruptures or spills preoperatively or intraoperatively.
Regional lymph nodes are involved.
Stage IV: Distant bone or lung metastasis is present.